nach oben

Erschienen in:

01.07.2015 | Review

Distinguishing Susac’s syndrome from multiple sclerosis

verfasst von: Katherine A. Buzzard, Stephen W. Reddel, Con Yiannikas, D. Sean Riminton, Michael H. Barnett, Todd A. Hardy

Erschienen in: Journal of Neurology | Ausgabe 7/2015

Einloggen, um Zugang zu erhalten

Abstract

Susac’s syndrome is considered a rare differential diagnosis in the work-up of suspected multiple sclerosis. Over the last decade or so, significant advances in our understanding of Susac’s syndrome mean that it can now be readily distinguished from multiple sclerosis in the majority of cases with a careful history and close attention to MR imaging. Supporting investigations such as CSF examination, fluorescein angiogram, visual field perimetry and audiology often yield important clues. In milder cases, however, diagnostic uncertainty can exist, and forme frustes of the disease are recognised. In this article, we discuss the clinical features of Susac’s syndrome with emphasis on the findings and investigations that help to differentiate it from MS.

Susac JO, Hardman JM, Selhorst JB (1979) Microangiopathy of the brain and retina. Neurology 29(3):313–316CrossRefPubMed

Susac J (1994) Susac’s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 44:591–593CrossRefPubMed

Hardy TA (2013) New developments in Susac’s syndrome. Adv Clin Neurosci Rehabil 13(1):6–8

O’Halloran HS, Pearson PA, Lee WB, Susac JO, Berger JR (1998) Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature. Ophthalmology 105(6):1038–1044CrossRefPubMed

Rennebohm R, Egan R, Susac J (2008) Treatment of Susac’s syndrome. Curr Treat Options Neurol 10(67–74):12

Rennebohm R, Susac JO, Egan RA, Daroff RB (2010) Susac’s Syndrome–update. J Neurol Sci 299(1–2):86–91CrossRefPubMed

Nazliel B, Akyol A, Zeynep Batur Caglayan H, Yildirim-Capraz I, Irkec C (2014) Susac’s syndrome in a patient diagnosed with MS for 20 Years: a case report. Case Rep Neurol Med 214648

Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T, Aktas O, Ringestein EB, Paul F, Kleffner I (2013) Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol 9(6):307–316CrossRefPubMed

Orton SM, Herrera BM, Yee IM, Valdar W, Ramagopalan SV, Sadovnick AD, Ebers GC, Canadian Collaborative Study Group (2006) Sex ratio of multiple sclerosis in Canada: a longitudinal study. Lancet Neurol 5(11):932–936CrossRefPubMed

10.

Goodin DS (2014) The epidemiology of multiple sclerosis: insights to disease pathogenesis. Handb Clin Neurol 122:231–266CrossRefPubMed

11.

Milo R, Kahana E (2010) Multiple sclerosis: geoepidemiology, genetics and the environment. Autoimmun Rev 9(5):A387–A394CrossRefPubMed

12.

Fischer M, Kunkel A, Bublak P, Faiss JH, Hoffmann F, Sailer M, Schwab M, Zettl UK, Köhler W (2014) How reliable is the classification of cognitive impairment across different criteria in early and late stages of multiple sclerosis? J Neurol Sci 343(1–2):91–99CrossRefPubMed

13.

Monteiro ML, Swanson RA, Coppeto JR, Cuneo RA, DeArmond SJ, Prusiner SB (1985) A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions. Neurology 35(8):1113–1121CrossRefPubMed

14.

Francis HW, Makary C, Halpin C, Crane BT, Merchant SN (2011) Temporal bone findings in a case of Susac’s syndrome. Otol Neurotol 32(8):1198–1204PubMedCentralCrossRefPubMed

15.

Saw VP, Canty PA, Green CM, Briggs RJ, Cremer PD, Harrisberg B, McCluskey P, O’Day J, Paine M, Wakefield D, Watson JD (2000) Susac syndrome: microangiopathy of the retina, cochlea and brain. Clin Experiment Ophthalmol 28(5):373–381CrossRefPubMed

16.

Ramanathan S, Lenton K, Burke T, Gomes L, Storchenegger K, Yiannikas C, Vucic S (2013) The utility of multimodal evoked potentials in multiple sclerosis prognostication. J Clin Neurosci 20(11):1576–1581CrossRefPubMed

17.

Toosy AT, Mason DF, Miller DH (2014) Optic neuritis. Lancet Neurol 13(1):83–99CrossRefPubMed

18.

Gordon LK, Goldstein DA (2014) Gender and uveitis in patients with multiple sclerosis. J Ophthalmol 2014:565262PubMedCentralCrossRefPubMed

19.

Brandt AU, Zimmermann H, Kaufhold F, Promesberger J, Schippling S, Finis D, Aktas O, Geis C, Ringelstein M, Ringelstein EB, Hartung HP, Paul F, Kleffner I, Dörr J (2012) Patterns of retinal damage facilitate differential diagnosis between Susac syndrome and MS. PLoS One 7(6):e38741PubMedCentralCrossRefPubMed

20.

Swingler RJ, Compston DA (1992) The morbidity of multiple sclerosis. Q J Med 83(300):325–337PubMed

21.

Lublin FD, Reingold SC (1996) Defining the clinical course of multiple sclerosis: results of an international survey. National Multiple Sclerosis Society (USA) Advisory Committee on clinical trials of new agents in multiple sclerosis. Neurology 46(4):907–911CrossRefPubMed

22.

Rovaris M, Confavreux C, Furlan R, Kappos L, Comi G, Filippi M (2006) Secondary progressive multiple sclerosis: current knowledge and future challenges. Lancet Neurol 5(4):343–354CrossRefPubMed

23.

Lublin FD, Reingold SC, Cohen JA, Cutter GR, Sørensen PS, Thompson AJ, Wolinsky JS, Balcer LJ, Banwell B, Barkhof F, Bebo B Jr, Calabresi PA, Clanet M, Comi G, Fox RJ, Freedman MS, Goodman AD, Inglese M, Kappos L, Kieseier BC, Lincoln JA, Lubetzki C, Miller AE, Montalban X, O’Connor PW, Petkau J, Pozzilli C, Rudick RA, Sormani MP, Stüve O, Waubant E, Polman CH (2014) Defining the clinical course of multiple sclerosis: the 2013 revisions. Neurology 83(3):278–286PubMedCentralCrossRefPubMed

24.

Rennebohm RM, Susac JO (2007) Treatment of Susac’s syndrome. J Neurol Sci 257(1–2):215–220CrossRefPubMed

25.

Jarius S, Kleffner I, Dörr JM, Sastre-Garriga J, Illes Z, Eggenberger E, Chalk C, Ringelstein M, Aktas O, Montalban X, Fechner K, Stöcker W, Ringelstein EB, Paul F, Wildemann B (2014) Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study. J Neuroinflammation 11(1):46PubMedCentralCrossRefPubMed

26.

Feresiadou A, Eriksson U, Larsen HC, Raininko R, Nygren I, Melberg A (2014) Recurrence of Susac syndrome following 23 Years of remission. Case Rep Neurol 6(2):171–175PubMedCentralCrossRefPubMed

27.

Jarius S, Neumayer B, Wandinger K, Hartmann M, Wildemann B (2009) Anti-endothelial serum antibodies in a patient with Susac’s syndrome. J Neurol Sci 285:259–261CrossRefPubMed

28.

Hardy TA, O’Brien B, Gerbis N, Barnett MH, Reddel SW, Brewer J, Herkes GK, Silberstein P, Garsia RJ, Watson JDG, Gupta R, Parratt JD, Buckland ME (2014) Brain histopathology in three cases of Susac’s syndrome: implications for lesion pathogenesis and treatment. J Neurol Neurosurg Psychiatry. doi:10.1136/jnnp-2014-308240. [Epub ahead of print]

29.

Fox RJ, Costello F, Judkins AR, Galetta SL, Maguire AM, Leonard B, Markowitz CE (2006) Treatment of Susac syndrome with gamma globulin and corticosteroids. J Neurol Sci 251(1–2):17–22CrossRefPubMed

30.

Petty GW, Engel AG, Younge BR, Duffy J, Yanagihara T, Lucchinetti CF, Bartleson JD, Parisi JE, Kasperbauer JL, Rodriguez M (1998) Retinocochleocerebral vasculopathy. Medicine (Baltimore) 77(1):12–40CrossRef

31.

Magro CM, Poe JC, Lubow M, Susac JO (2011) Susac syndrome: an organ-specific autoimmune endotheliopathy syndrome associated with anti-endothelial cell antibodies. Am J Clin Pathol 136(6):903–912CrossRefPubMed

32.

Barnett MH, Prineas JW (2004) Relapsing and remitting multiple sclerosis: pathology of the newly forming lesion. Ann Neurol 55(4):458–468CrossRefPubMed

33.

Lucchinetti C, Brück W, Parisi J, Scheithauer B, Rodriguez M, Lassmann H (2000) Heterogeneity of multiple sclerosis lesions: implications for the pathogenesis of demyelination. Ann Neurol 47(6):707–717CrossRefPubMed

34.

Trapp BD, Bö L, Mörk S, Chang A (1999) Pathogenesis of tissue injury in MS lesions. J Neuroimmunol 98(1):49–56CrossRefPubMed

35.

Susac J, Murtagh F, Egan R, Berger J, Bakshi R, Lincoff N, Gean A, Galetta S, Fox R, Costello F, Lee A, Clark J, Layzer R, Daroff R (2003) MRI findings in Susac’s syndrome. Neurology 61:1783–1787CrossRefPubMed

36.

Horowitz AL, Kaplan RD, Grewe G, White RT, Salberg LM (1989) The ovoid lesion: a new MR observation in patients with multiple sclerosis. Am J Neuroradiol 10(2):303–305PubMed

37.

Gean-Marton AD, Vezina LG, Marton KI, Stimac GK, Peyster RG, Taveras JM, Davis KR (1991) Abnormal corpus callosum: a sensitive and specific indicator of multiple sclerosis. Radiology 180(1):215–221CrossRefPubMed

38.

Kim SS, Richman DP, Johnson WO, Hald JK, Agius MA (2014) Limited utility of current MRI criteria for distinguishing multiple sclerosis from common mimickers: primary and secondary CNS vasculitis, lupus and Sjogren’s syndrome. Mult Scler 20(1):57–63CrossRefPubMed

39.

Kleffner I, Deppe M, Mohammadi S, Schwindt W, Sommer J, Young P, Ringelstein EB (2010) Neuroimaging in Susac’s syndrome: focus on DTI. J Neurol Sci 299(1–2):92–96CrossRefPubMed

40.

Wuerfel J, Sinnecker T, Ringelstein EB, Jarius S, Schwindt W, Niendorf T, Paul F, Kleffner I, Dörr J (2012) Lesion morphology at 7 Tesla MRI differentiates Susac syndrome from multiple sclerosis. Mult Scler 18(11):1592–1599CrossRefPubMed

41.

Bot JC, Barkhof F, à Nijeholt GL, van Schaardenburg D, Voskuyl AE, Ader HJ, Pijnenburg JA, Polman CH, Uitdehaag BM, Vermeulen EG, Castelijns JA (2002) Differentiation of multiple sclerosis from other inflammatory disorders and cerebrovascular disease: value of spinal MR imaging. Radiology 223(1):46–56CrossRefPubMed

42.

Hua le H, Donlon SL, Okuda DT (2014) A case of Susac syndrome with cervical spinal cord involvement on MRI. J Neurol Sci 337(1–2):228–231CrossRef

43.

Allmendinger AM, Mallery RM, Magro CM, Wang N, Egan RA, Samuels MA, Callahan A, Viswanadhan N, Klufas RA, Hsu L, Prasad S (2014) Cauda equina involvement in Susac’s syndrome. J Neurol Sci 337(1–2):91–96CrossRefPubMed

44.

Awad A, Hemmer B, Hartung HP, Kieseier B, Bennett JL, Stuve O (2010) Analyses of cerebrospinal fluid in the diagnosis and monitoring of multiple sclerosis. J Neuroimmunol 219(1–2):1–7CrossRefPubMed

45.

Cross AH, Wu GF (2010) Multiple sclerosis: oligoclonal bands still yield clues about multiple sclerosis. Nat Rev Neurol 6(11):588–589CrossRefPubMed

46.

Belizna C, Tervaert JW (1997) Specificity, pathogenicity, and clinical value of antiendothelial cell antibodies. Semin Arthritis Rheum 27(2):98–109CrossRefPubMed

47.

Lee M, Amezcua L (2009) Treatment of Susac’s syndrome with rituximab: a case report. Neurology 72:A203CrossRef

48.

Hardy TA, Garsia RJ, Halmagyi GM, Lewis SJ, Harrisberg B, Fulham MJ, Barnett MH (2011) Tumour necrosis factor (TNF) inhibitor therapy in Susac’s syndrome. J Neurol Sci 302(1–2):126–128CrossRefPubMed

49.

Buzzard KA, Broadley SA, Butzkueven H (2012) What do effective treatments for multiple sclerosis tell us about the molecular mechanisms involved in pathogenesis? Int J Mol Sci 13(10):12665–12709PubMedCentralCrossRefPubMed

50.

Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO (2008) Aggressive immunosuppressive treatment of Susac’s syndrome in an adolescent: using treatment of dermatomyositis as a model. Pediatr Rheumatol Online J 29(6):3CrossRef

51.

Laird PW, Newman NJ, Yeh S (2012) Exacerbation of Susac syndrome retinopathy by interferon Beta-1a. Arch Ophthalmol 130(6):804–806CrossRefPubMed

52.

Lenercept Multiple Sclerosis Study Group (1999) TNF neutralization in MS: results of a randomized, placebo-controlled multicenter study. The Lenercept Multiple Sclerosis Study Group and The University of British Columbia MS/MRI Analysis Group. Neurology 53:457–465CrossRef

53.

Barnett MH, Hardy TA, Garsia RJ, Halmagyi GM, Lewis SJ, Harrisberg B, Fulham MJ (2012) Susac’s syndrome. J Neurol Sci 314(1–2):183CrossRef

Titel: Distinguishing Susac’s syndrome from multiple sclerosis
verfasst von: Katherine A. Buzzard
Stephen W. Reddel
Con Yiannikas
D. Sean Riminton
Michael H. Barnett
Todd A. Hardy
Publikationsdatum: 01.07.2015
Verlag: Springer Berlin Heidelberg
Erschienen in: Journal of Neurology / Ausgabe 7/2015
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-014-7628-9

Leitlinien kompakt für die Neurologie

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Neu im Fachgebiet Neurologie

Hirnblutung unter DOAK und VKA ähnlich bedrohlich

17.05.2024 Direkte orale Antikoagulanzien Nachrichten

Kommt es zu einer nichttraumatischen Hirnblutung, spielt es keine große Rolle, ob die Betroffenen zuvor direkt wirksame orale Antikoagulanzien oder Marcumar bekommen haben: Die Prognose ist ähnlich schlecht.

Thrombektomie auch bei großen Infarkten von Vorteil

16.05.2024 Ischämischer Schlaganfall Nachrichten

Auch ein sehr ausgedehnter ischämischer Schlaganfall scheint an sich kein Grund zu sein, von einer mechanischen Thrombektomie abzusehen. Dafür spricht die LASTE-Studie, an der Patienten und Patientinnen mit einem ASPECTS von maximal 5 beteiligt waren.

Schwindelursache: Massagepistole lässt Otholiten tanzen

14.05.2024 Benigner Lagerungsschwindel Nachrichten

Wenn jüngere Menschen über ständig rezidivierenden Lagerungsschwindel klagen, könnte eine Massagepistole der Auslöser sein. In JAMA Otolaryngology warnt ein Team vor der Anwendung hochpotenter Geräte im Bereich des Nackens.

Schützt Olivenöl vor dem Tod durch Demenz?

10.05.2024 Morbus Alzheimer Nachrichten

Konsumieren Menschen täglich 7 Gramm Olivenöl, ist ihr Risiko, an einer Demenz zu sterben, um mehr als ein Viertel reduziert – und dies weitgehend unabhängig von ihrer sonstigen Ernährung. Dafür sprechen Auswertungen zweier großer US-Studien.

Update Neurologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Die Highlights vom Kongress des American College of Cardiology 2024

Springer Medizin

Abstract

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 7/2015

Acknowledgement to Reviewers 2014

Acyclovir-related kidney injury during alemtuzumab infusion

Isolated tumefactive demyelinating lesions: diagnosis and long-term evolution of 16 patients in a multicentric study

Walking function in clinical monitoring of multiple sclerosis by telemedicine

Improving survival in amyotrophic lateral sclerosis: future treatments in a modern service